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Unilateral renal cystic disease (URCD) is a multicystic disease, characterized by varying sized cysts localized in a diffusely enlarged kidney without forming a distinct encapsulated mass. Except for its unilaterality, gross and histological findings of URCD are indistinguishable from those of autosomal dominant polycystic kidney disease (ADPKD). However, unlike ADPKD, URCD patients show neither a genetic background nor progressive deterioration in renal function; thus, the differential diagnosis of URCD from ADPKD is important, especially from the genetic and prognostic standpoint. Usually, URCD patients have no cardiac valvular abnormalities and no cystic diseases in other intraperitoneal organ, except simple cyst. Intracranial aneurysm can be found in 5-10% of ADPKD patients, but not in previous reported fourteen URCD cases yet. We report a 41year-old patient with URCD and cerebral artery aneurysm. Careful family history taking and ultrasonographic examination of his parents and siblings revealed that they have no renal cystic disease. We followed the patient for 13 months and his renal function did not deteriorate. (Korean J Nephrol 2004;23(3):496-499)
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